Clinical Characterization Of41Children With Noncompaction Ventricular Myocardium

Background：Noncompaction of ventricular myocardium (NVM), known as spongymyocardium or persistent sinusoids, is reported as a genetic disease thatcharacterized by excessively prominent trabeculations and deepintertrabecular recesses. It is reported that refractory heart failure, arrythmiaand embolic events were the main performance clinically.In the past, it isbelieved that NVM has a poor prognosis, but recent reports draw a differentconclusion, especially different from adult and children.Object：The purpose of this study is to investigate the clinical characteristics，outcome and influence factors in patients with NVM through clinical data ofthe41children.Method：We collected the clinical data of the41patients hospitalized fromSeptember2007to January2014in Chongqing Medical University affiliated children’s hospital. A retrospective study was performed according tomedical history of patients, clinical manifestation, imaging examination,laboratory examination, and so on. We also completed follow-up andanalysis of related factors affecting the prognosis of NVM.Results:The41patients considered with NVM consists of18males and23females, moreover,23children were less than1year old.31chaildren hadvarying degree of heart failure,account for75.6%of all patients. It iscommon to see ECG changes,especially rapid arrhythmia(43.9%). Of the41patients,17are isolated noncompaction of ventricular myocardium(41.5%),9children with other different types of myocardium(5with dilatedcardiomyopathy and4with endocardium fibrosis myopathy),17withcongenital heart disease(41.5%).The most frequent Congenital heart diseasewith NVM are Patent ductus arteriosus,Atrial septal defect and Ventricularseptal defect. The mean follow-up time is9.7±12.6months,13patients losecontact,12patients die,and16patients alive.In the12die cases,the meanonset-die time is2.5±2.1months,and7were less than1year old(58.3%).Wemade the Kaplan-Meier surve curve, it shows that the1year survival rate ofNVM is64.4%.We also anaysis the possible risk factors,it turned out thatrapid arrhythmia,Creatine kinase and Creatinine are statistically significant（P<0.05）,beyond that, NC/Cisthepossibleriskfactor（P=0.053）and theCreatinine is an independent predictors of poor outcome. ConclusionThe main clinical manifestation are cardiac insufficiency andarrhythmia. Thrombosis and embolism events are rare.NVM is a diseasewith poor prognosis,and the refractor heart failure and malignant arrhythmiaare the most common cause of death.Patients surviving1year afterpresentation had a higher conditional survival. Therefore, early and activetreatment may control patient’s condition deteriorated and improve theprognosis.